Do you think your little child is slowly growing legs or arms? Perhaps he has Olier’s disease. This is a disease in which abnormalities in the development of the bone skeleton occur, that is, its dysplasia. Other names for this disease are multiple bone chondromatosis, unilateral chondromatosis or bone dyschondroplasia. The disease was given its main name on behalf of the Lyon surgeon Olga. He discovered and described the disease through the use of X-rays in 1899.
What is Olier disease
This disease refers to abnormalities of the bone skeleton, to which many diseases belong, including hip dysplasia, well known to many mothers of babies.
The disease often manifests itself in childhood, due to which it was previously believed that this is a disease of children. It has now been proven that pathology can also debut in adults (signs can occur even at the age of 20-40). This is a congenital pathology, but it is detected after some time - in early childhood or in adolescence - that is, during intensive growth, when bone deformations and / or abnormal development of arms and legs become apparent.
In Oliver's disease, in the metaphyses of the long tubular bones of the limbs, cartilage growths appear. The disease develops mainly in the bones, cartilage and joints of the hands and feet, in particular in the feet and hands, as well as in the bones of the pelvis, but it also occurs in the ribs, sternum or skull.
The growths, or enchondromas, make the outer layer of the bone thin and brittle. In fact, these are benign tumors that can develop into malignant (for example, chondrosarcomas). Fortunately, this is extremely rare.
During puberty, growths are stabilized and replaced by bones.
So, Olier disease is a serious hereditary disease, ossification of the cartilage skeleton, in which there is also a thickening of the metaphyses (neck of the tubular bones). The cause of its occurrence is still unknown. In some cases, it is of an autosomal dominant genetic nature.
Diseases similar to dyschondroplasia are rare Muffucci and Proteus syndromes . At the first, in addition to benign cartilaginous growths and skeletal deformities, hemangiomas are formed on the skin - dark red spots of irregular shape, consisting of blood vessels. In the second, benign tumors develop in various parts of the body.
Who is affected
Bone chondromatosis was previously thought to be rare. But now, when the X-ray study is conducted quite widely, it turned out that it can not be called a rarity. If 20 cases were described 20 years ago, now they are twice as many.
It is usually impossible to determine the exact beginning if regular dynamic research using X-rays was not carried out from birth, especially in early childhood and intrauterine development, when, in fact, Olier’s disease occurs. This disease, by the way, is detected in girls almost twice as often as in boys.
Symptoms of the disease
In order not to miss the signs of the disease, parents should be wary if the child:
- some parts of the limb / limbs are delayed in development;
- asymmetry and shortening are noted in them;
- lameness is observed;
- there is a skew of the pelvis;
- there are joint distortions of the valgus or varus type.
All these factors make it possible to assume that the child has Olée’s disease.
The disease does not always appear immediately at birth, but already at the age of 1-4 years you can see that the child’s legs or arms grow slower than expected. This is the main symptom of the disease, which can not be ignored. Otherwise, the baby develops normally, despite the fact that he is diagnosed with Olée’s disease.
Dyschondroplasia most often affects one limb, however, cases of damage to both are common. If the disease touches the legs, the child will be short due to their shortening. Especially strongly lag behind in bone growth, if the pathological process began very early. Deformations proceed painfully, progress slowly. A limb susceptible to a disease becomes shorter, thicker, gait changes, lameness is observed. Varus or valgus deformity of the knee (less commonly the proximal end of the thigh or foot), scoliosis of the spine can be observed. Muscular atrophy is not present in this disease.
The phalanges, metatarsals, and, in some cases, metacarpals , undergo large changes . They are shortened, enlarged, clumsy and contain many cartilaginous enlightenments.
Olie’s sufferers are susceptible to fractures, because due to the disease they develop bone tissue incorrectly. However, fractures often heal without problems.
Accompanying illnesses
Dyschondroplasia can be accompanied by damage to other organs, although concomitant diseases do not always develop. In particular, in young girls, together with Olier disease, ovarian teratoma and endocrine disorders can be observed, including severe ones, including very early menstruation and premature puberty with acceleration of ossification.
However, the development returns to normal when the ovarian tumor is removed.
Diagnostics
To detect Olée’s disease, it is first necessary to take an X-ray. A bone biopsy and magnetic resonance imaging should also be performed. If a disease is detected, patients need a regular examination by a doctor in order to timely detect malignant transformations into chondrosarcoma, if necessary.
Differential diagnosis
Differential diagnosis of Olier disease, in fact, is not needed after an X-ray examination, since the picture of chondromatosis is very specific. Nevertheless, each case is individual, and the set of painful signs is practically not repeated, therefore, doctors should exclude a number of similar diseases.
X-rays exclude atypical chondrodystrophy, hemihypertrophy or hematrophy of bones, rickets and some other diseases. Also, thanks to x-rays, Recklingausen's disease is excluded, which can usually be confused with Ollier's disease. The main principle - it is necessary to make an x-ray of the entire skeleton, and not part of it.
Treatment principles
Unfortunately, dyschondroplasia currently has no rational treatment. Unlike, for example, a disease such as hip dysplasia, chondromatosis is treated only with surgical correction of the deformity. In some cases, artificial endoprosthetics are indicated. The rest of the treatment that the patient should receive is symptomatic and supportive.
Surgical intervention is indicated mainly for adults, since, according to modern observations, no lasting results were obtained with surgical correction at school age, which means that it makes sense to carry out surgical treatment only with formed deformations, but not dynamic ones. However, with severe lesions, surgical intervention is also possible for children, especially when it comes to fingers, which are significantly bent in Oliver's disease.
The disease requires the maintenance of joints and bones: patients are shown wearing orthopedic shoes, using an orthopedic apparatus and other devices to fix the joints. Patients must necessarily engage in exercises designed specifically for this pathology of the physical therapy complex.
Gymnastics exercises and exercises on special simulators are also useful. This helps restore muscle tone and strengthen ligaments.
Forecast
According to studies, most often by the puberty age, cartilage is replaced by bone (perhaps this is why adult cases of the disease are extremely rare). However, in young children, the prognosis is less rosy: deformation, although slowly, is progressing. This is precisely what Olie’s disease is dangerous.
The disease is so individually manifested in each case that the overall prognosis is clearly difficult to assess. With numerous lesions, it is more favorable than in the case of localized single changes. Single deformations lead to the shortening of one of the limbs and, consequently, to their asymmetry, especially when it comes to young children. And this is already impossible to fix. Also, with the early development of tumors, the risk of various finger deformities increases.
Chondrosarcoma Transformation
Despite the fact that transformation into a malignant species - chondrosarcoma - occurs infrequently, there is still a chance of developing cancer. Mostly this happens in adolescence, although a number of modern studies show that this can happen much later. For this reason, patients with dyschondroplasia are recommended regular medical consultations and medical examinations.